If you’re a woman living with pulmonary hypertension and you’re thinking about starting a family, it’s important to have a conversation with your pulmonary hypertension specialist and your primary care physician. PH experts recommend that PH patients avoid becoming pregnant due to the dangerous risks it poses to both mother and child. The information below is adapted from a consensus statement in the USA (see birth control) issued by the Pulmonary Hypertension Association’s Scientific Leadership Council as well as recommendations provided by a panel of PH-treating medical professionals at PHA’s 2008 International PH Conference. This information is no substitute for a personal discussion with your PH specialist and your primary care physician about the specifics of your situation, but meant to answer some basic questions about this difficult issue (shared with you from the PHA USA website phassociation.org).

Why did my doctor advise me to avoid pregnancy?

Carrying a child can be dangerous for PH patients due to the increased strain it places on the heart and lungs. In a normal pregnancy, your blood volume increases by about 50%. The sudden change in blood volume during and after delivery can lead to right-heart failure in PH patients whose right-heart is already overworked due to the increased pressure in their pulmonary arteries. Estimates place the risk of pregnancy-related heart failure in PH patients at 30-50%. Some of the medications prescribed to PH patients are also known to be harmful to the developing fetus.

What type of birth control should I consider?

PH specialists recommend that sexually active women with PH use at least two and preferably three forms of birth control. Doctors do not encourage hormonal contraceptives, including the birth control pill and the birth control patch, because they can increase your risk of blood clots. Certain PH medications may also decrease their effectiveness. Instead, PH patients should talk to their doctors about barrier methods (condoms and diaphragms with spermicide), estrogen-free products, and surgical options (tubal ligation for women, or a vasectomy for your male partner if you’re in a monogamous relationship).

What do I do if I become pregnant?If you do become pregnant, make an appointment with your PH specialist as immediately.

I want to have children. What are my options?

While starting a family can be challenging for women with PH, it is not impossible. Once you reach the place where you can accept that you won’t be able to carry a child of your own, you can begin to research your best available options. You might consider foster parenting or adoption. While adoption agencies screen carefully for health and financial stability, adoption is not out of the question for patients who are in stable health and have a strong family support network. If you and your PH specialist and your primary care physician feel you’re healthy enough to consider it, you can ask your PH specialist to write your adoption agency a letter of support to help the process along. (Read more under A- adoption option).

The use of surrogate mothers (women who have fertilized eggs inserted into their uterus to carry a fetus) is an option that some PH specialists have considered for selected patients, although this has been done in only a few instances. There are ethical, practical and medical issues associated with the use of surrogate mothers for PH patients that should be discussed not only with your PH specialist and your primary care physician but also with an experienced reproductive counselor.

Where can I find more information?See the article below: from PHA's International PH Conference in 2008 to learn more about issues relevant to young women with PH, including pregnancy and birth control or listen online at www.PHassociation.org

Another fact about pregnancy is that when you are informed you cannot have children, if you don't already have some or you were hoping for more, it can be literally devastating. You may experience a range of emotions from anger, disbelief, guilt and grief.

​You may not be able to be around your friends who have children because it's to painful. Or you may be ok and you may think of fostering or adopting if your health is stable enough. It is possible and it does happen. The important thing is that you speak to a health professional about these feelings and as a couple you get counselling to deal with the news and how you are going to cope with it. Some of the PH treatment centres may have Psychologists attached to them so ask for a referral or seek an independent doctor you are comfortable with.

Birth control and hormonal therapy in pulmonary hypertension - Consensus Statement Issued by the Scientific Leadership Council USA

DISCLAIMER: This information is for general information only. These guidelines may not apply to your individual situation. You should rely on the information and instructions given specifically to you by your PH specialist and/or the nurses at your PH Center. This information is general in nature and may not apply to your specific situation. It is not intended as legal, medical or other professional advice, and should not be relied upon as a substitute for consultations with qualified professionals who are familiar with your individual needs.

The risk of pregnancy-related death in women with PAH is substantial, with risk of dying reported to be 30 to 50%. For this reason alone, pregnancy is contraindicated in patients with PAH. In addition to the hemodynamic load of pregnancy, which means that your heart has to work extra, medications used for PAH are teratogenic (harmful) to the developing fetus, including warfarin (Coumadin); endothelin receptor antoagonists (Bosentan and ambrisentan) and aldactone (which may be used by PAH patients), and further add to the risk of pregnancy. Because of the risk to both the patient and the fetus, use of some form of birth control and avoidance of pregnancy is strongly advised in women of childbearing age with PAH.

There are no published guidelines for birth control use in PAH, and there is no consensus regarding the best form of birth control. The two safest methods of birth control are 1) the barrier method, which may include condoms in men and/or a diaphragm with spermicide in women, and 2) a vasectomy in the male partner for a woman with PAH in a monogamous (one partner) relationship. The failure rate in preventing pregnancy with barrier methods, when used properly, is quite low. Tubal ligation was felt by many PH specialists to be an acceptable option in patients who were not in severe heart failure. In the sickest patients, consideration of other birth control methods is recommended.

In a survey of 23 PH specialists from North America and Europe, the majority felt that use of estrogen-containing birth control pills (BCP) was acceptable as long as patients were anti coagulated with warfarin. Birth control pills containing the lowest amount of estrogen are recommended. However, nearly half of the specialists did not advocate using BCP for their patients, and some actively discouraged patients from doing so because of concern over the possible role of estrogen in worsening PAH. Sixty-five percent of specialists indicated that use of hormonal therapy in post-menopausal women with PAH was acceptable. Progesterone alone, taken orally (by mouth), can be as effective as estrogen in combination with progesterone in preventing pregnancy.

However, when using progesterone alone, the pills must be taken at the same time every day to be effective. Intramuscular injections of progesterone (Depo-Provera) are another option; however, this is often accompanied by fluid retention, which may be harmful to patients with right heart failure. There is also a 20 to 30% incidence of irregular, heavy menstrual bleeding associated with the use of progesterone injections that may be worsened by the use of warfarin. Furthermore, intramuscular injections may be associated with localized bleeding in patients receiving warfarin.

A potential interaction exists between endothelin receptor antagonists such as Bosentan (Tracleer) and hormonal birth control, although no studies have demonstrated this to date. Women should not rely on hormonal birth control alone when taking Bosentan. Also, many antibiotics can decrease the effectiveness of the hormonal forms of birth control. Thus, if a woman with PAH requires antibiotics, an additional method of contraception should be used during the entire menstrual cycle until the next period occurs.

Intrauterine devices (IUDs) can be used for birth control, although this is not recommended as the first choice for birth control. Traditional IUDs may be associated with excessive bleeding at the time of menses that may be exacerbated by the use of warfarin. The device should be removed if excessive bleeding occurs.

A newer IUD that releases progesterone (Mirena) has a lower risk of bleeding (and has been used to treat excessive menstrual bleeding) and may be an option for some patients. Prophylactic antibiotic use should be used at the time of insertion and removal in most patients with PAH related to congenital heart disease.

The use of surrogate mothers (women who have fertilized eggs inserted into their uterus to carry a fetus) is an option that some PH specialists have considered for selected patients, although this has been done in only a few instances. There are many ethical, practical and medical issues associated with the use of surrogate mothers for PAH patients that should be discussed not only with treating physicians but also with experienced reproductive counselors and often with a patient’s religious leader

(This information was shared with you from PHA USA phassociaton.org).

Pregnancy and PH- What you need to know (shared with you through pulmonaryhypertensionnews.com) 2016

​Pulmonary hypertension (PH) is a rare, life-threatening disease that affects the pulmonary arteries, the lungs, and the heart. Patients who suffer from the disease have narrowed and obstructed pulmonary arteries, which are the vessels responsible for transporting blood from the heart to the lungs. As a result, the heart has to work harder to properly pump blood and becomes overloaded with work, resulting in an enlargement and weakening of the organ. In severe cases, pulmonary hypertension can cause the total blockage of the pulmonary arteries and right heart failure, when the right ventricle loses its capacity to pump blood to the lungs.

During pregnancy, women’s blood volume increases by between 30 and 50 percent compared to its normal amount in order to nourish the baby. This increase in the amount of blood that needs to be pumped from the heart creates increased stress on the organ and circulatory system. Every pregnant woman’s heart — even those who are completely healthy — becomes overloaded with work during the pregnancy and even more so during labor and delivery. In a patient who suffers from pulmonary hypertension and whose heart is already under stress, the risks are particularly high and may result in the death of the mother.

There are options available to you and your partner that you may wish to consider to do with fostering or adopting a child. The first step is to discuss this with your PH Doctor to make sure they feel you are stable enough to cope with this commitment.

Links to provide you with various information on this topic within Western Australia

My patients ask me all sorts of questions– how am I doing, is my PAH getting better, what activities can I do and what should I avoid. In more than 14 years of caring for PAH patients I can remember only one patient asking about sexual activity. There is a paucity of published information and patients are often embarrassed or shy when it comes to asking. Nonetheless, just about all of my patients are curious, scared and desperate for accurate information. As a result over the years I have come to include a discussion about sex in my standard educational talks with my patients. Let’s face it, sex is an important part of life and having a chronic illness does not change that.

The Basics - Rule number one: Women with pulmonary hypertension should not get pregnant. Some studies have reported up to 50% maternal mortality in patients with severe PAH. So any discussion about sex must start with defining effective contraception. All female patients of child bearing potential (that means if you have a uterus and are less than 55) must use reliable contraception. In general we prefer to avoid estrogen containing pills. Depot-Provera is a great choice. Condoms when used from start to finish not just prevent pregnancy but also prevent sexually transmitted diseases. An IUD (this is a small device the gynecologist inserts through the vagina and cervix into the uterus and remains in place for 5- 10 years) is a great option for women in long-term relationships.

Rule number two: Listen to your body. During sex your heart rate increases, your blood pressure increases, and blood flow is redirected. The magnitude of these changes depends on how “athletic” you and your partner are, and very importantly whether you are with a new partner or someone you have been with for years. A good strategy is to go slow. If you feel light headed, short of breath or develop chest pain, take a break. In the beginning let your partner do more of the “work”.

How do I know if I am well enough for sex? First, chronic illness reduces your libido. So as you start regaining an interest in sex it generally means that your PAH is improving. In general if you can walk through the grocery store, take a shower and get dressed without having to stop then you are probably ok. Patients with marked shortness of breath or symptoms with minimal activity should wait.

Do PAH medications affect a man’s ability to father a child? Most of the PAH medications don’t affect sperm counts or sperm motility. However, the endothelin receptor antagonists such as Bosentan (Tracleer) and Ambrisentan (Letairis) may reduce sperm counts.

Are there things that I should avoid? In general patients with PAH tolerate having their head below their heart poorly. So keep this in mind as you choose your positions. Combining alcohol with PAH medications is never a good idea.

My goal in caring for patients with PAH is to restore them to the best health I can and to give them the best quality of life. Part of a good quality of life is a healthy sex life. Ask your PAH team questions about sex and don’t be shy in discussing birth control/contraception with your team.

Pregnancy and Pulmonary Hypertension Part 1

There are many challenges in caring for patients with PAH. We ask our patients to take many different medications to help treat their disease process and we ask them to put up with side effects from these medications. We ask our patients to change their lifestyles and eating habits. One of the greatest challenges is the issue of pregnancy. PAH is particularly common in young women who might otherwise consider either starting a family or adding to their family.

Why is Pregnancy Harmful to a Woman with Pulmonary Arterial Hypertension?Understanding the normal changes to a woman’s body that take place during pregnancy helps us understand why carrying a pregnancy can be so dangerous. A woman’s body gradually retains salt and water during pregnancy such that by the end of the third trimester their blood volume has increased by 50%. There is an increase in 6 to 8 liters of water and about 1000meq of sodium. So if a woman had a total blood volume prior to pregnancy of 5 liters then at 36 weeks their blood volume would be 7.5 liters. Heart rate increases by 15 to 20 beats per minute and cardiac output increases up to 50%. This dramatic increase in blood volume is accompanied by a mild anemia. The anemia is well tolerated but in PAH the right ventricle is asked to work much harder. In a woman without pulmonary hypertension the heart has no problem meeting the increased work load that is required but in PAH the added fluid can result in progressive right heart failure and death.

In addition to the extra work that is required of the right ventricle in pregnancy, several of the medications that are commonly used to treat pulmonary arterial hypertension are teratogenic (very harmful to the growing fetus). Additional hurdles include the stress of delivery. Normal labor requires a woman to push very vigorously to help the baby exit the birth canal. This type of pushing can cause fainting and dangerously low blood pressure in a patient with PAH. Many women choose to have a “spinal”. This is an injection of medication to reduce the amount of pain and pressure experienced during the peak of labor. In patients with pulmonary hypertension, this spinal injection can precipitate life-threateningly low blood pressure. Cesarean section is sometimes required to safely deliver the baby. Options for making this comfortable include spinal injection of anesthesia or general anesthesia (being put to sleep). The latter can be very dangerous for PH patients even when performed by anesthesiologists expert in PAH management.

Pregnancy and Pulmonary Hypertension Part 2The literature has described the risk of pregnancy to the mother as very severe in patients with advanced PAH. Depending on how advanced the PAH, there may be a 50% to 80% chance of death. For patients with much milder PAH, the risks are much lower but still substantial. More recently as our treatment options for PAH patients have improved, the reported results have shown that in very carefully selected patients with well controlled pulmonary arterial hypertension, who are managed by doctors with tremendous expertise, pregnancy can be undertaken. So that everyone understands the key message here, pregnancy is always discouraged in patients with pulmonary arterial hypertension. However, recognizing that some women will wish to proceed with pregnancy regardless of the risks, it is imperative to find a PAH team that can help minimize the risks.

Discussing Pregnancy With Your PH TeamPregnancy in PAH patients should never be a surprise event. Sexually active women should be using highly effective birth control such as depot Provera, IUDs, or condoms with spermicide worn from start to finish. In select patients that have very mild PAH and are considering pregnancy, a discussion with their PAH physician should begin very early– long before you stop your birth control. The PH doctor will discuss with you the risks based on how you are doing. In my practice we would perform a right heart catheterization, an echo, blood tests and a six minute walk test to thoroughly understand how you are doing from a pulmonary hypertension perspective. Next we would have a very frank discussion where I would try and persuade you not to get pregnant. I would then send you to see a high risk obstetrician who would similarly try and persuade you to consider other options for becoming a parent such as adoption. If you were still intent on proceeding we would adjust your medications to remove the ones that are toxic to the fetus. We would also likely place you on continuous Prostanoid therapy such as subcutaneous Remodulin. We would see you frequently and meet regularly as a team with your high risk OB. In the third trimester we would see you weekly. We would formulate a plan for delivery with the OB team. We often prefer to induce delivery to avoid delivery when the team is not available. Delivery is a big deal. We typically have the pulmonary arterial hypertension ICU nurses, the PAH doctor, the OB doctor and nurses, and an anesthesiologist all present. There might be 10 people in the delivery room. After delivery the safest place is the PAH ICU not the normal post-delivery ward. The period of great risk to the mother extends for the first few weeks post-partum (after delivery).

What If A PH Patient Accidentally Becomes Pregnant?So what happens if you find that you are pregnant and it was not planned? This is a PH emergency. Immediately call your pulmonary hypertension doctor. After evaluating how you are doing from a PAH perspective and adjusting your medications, we would have a very frank conversation with you and your partner. The vast majority of the time, pregnancy termination is the correct course of action to prevent the mother from a high risk of dying during the pregnancy. This is always a very difficult decision.

Children born to women with late-stage gestational diabetes — caused by a pregnancy-related, transient increase in blood sugar levels (hyperglycemia) — or to women who are overweight or obese are known to be a higher risk of pulmonary complications, namely respiratory distress and persistent pulmonary hypertension of the newborn (PPHN). While efforts to treat diabetes during pregnancy are well-established, a new study suggests that circulating lipids generated by a mother’s high-fat diet are also important in creating this risk in an infant.​To better understand the possible association between obesity, diabetes, and PPHN, a research team at the University of South Dakota studied pregnant rats with gestational diabetes being fed a high-fat diet. They found that these rats gave birth to offspring with pulmonary complications, which lasted the three weeks they were alive. The data were published in the journal PloS One, in a study titled, “Consequences of a Maternal High-Fat Diet and Late Gestation Diabetes on the Developing Rat Lung.” “To date, there are very few studies investigating the effects of maternal HF diet on pulmonary development, and to our knowledge, no other study has looked at the combined effect or followed offspring past the perinatal time-point,” the team wrote. Researchers gave female rats a high-fat diet four weeks prior to mating. When the animals became pregnant, and eight days before delivery, the team induced diabetes in the animals. Because researchers wanted to exclude postnatal influence from diabetic or high-fat mothers, the pups were fed by normal foster mothers.

The team found that pregnant rats under a high-fat diet had an offspring mortality rate 89% higher than pregnant rats with diabetes or those given a healthier, control diet. Importantly, researchers found that high-fat diet combined with gestational diabetes affected lung maturity and lung function in the surviving three-week-old offspring. The combination reduced the lung’s ability to stretch and expand, known as lung compliance, in the young rats.

Based on lung vessel and alveolar morphological studies, the team proposed that impaired maturation and vascularization of the lung is probably multi factorial. Glucose, insulin, fatty acids, and inflammatory factors together seem to contribute to lung abnormalities.The authors concluded that the study may offer new therapeutic strategies to decrease lung complications in babies born to obese and diabetic mothers. “Knowledge gained provides a foundation for the investigation of preventative and therapeutic strategies aimed at decreasing pulmonary morbidity in at-risk infants. A desperate area in need of ongoing research is to continue to understand how a maternal HF [high-fat] diet could exacerbate the effects of diabetic pregnancy,” the authors wrote.

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